文献：内皮素B受体抗体简介说明

中文名称    内皮素B受体抗体    

英文名称    ETBR    

别    名    ABCDS; Ednra; Ednrb; EDNRB_HUMAN; Endothelin B receptor; Endothelin B receptor precursor; Endothelin receptor Non selective type; Endothelin receptor non-selective type; Endothelin receptor type B; ET B; ET-B; ET-BR; ETB; ETRB; Hirschsprung disease 2; HSCR; HSCR2; EDNRB; HSCR; WS4A.    

供 应 商    远慕生物

研究领域    心血管  免疫学  神经生物学  生长因子和激素      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 IF=1:100-500 内皮素B受体抗体（石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    50kDa    

细胞定位    细胞膜     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human EDNRB    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

保存条件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

内皮素B受体抗体产品介绍    background:

Endothelin receptor type B (ETRB) is a G protein-coupled receptor that activates a phosphatidylinoitol-calcium second messenger system. Its ligand, endothelin, consists of a family of three potent vasoactive peptides: ET1, ET2, and ET3. A splice variant (SVR), differing in the intracellular C terminal domain, has been described for this receptor. Both WT and the variant bind ET1. However, they exhibit different responses upon binding - suggesting that these two variants are functionally distinct.

Function:
Non-specific receptor for endothelin 1, 2, and 3. Mediates its action by association with G proteins that activate a phosphatidylinositol-calcium second messenger system.

Subcellular Location:
Cell membrane; Multi-pass membrane protein.

Tissue Specificity:
Expressed in placental stem villi vessels, but not in cultured placental villi smooth muscle cells.

Post-translational modifications:
Palmitoylation of Cys-402 was confirmed by the palmitoylation of Cys-402 in a deletion mutant lacking both Cys-403 and Cys-405.

DISEASE:
Defects in EDNRB are a cause of Waardenburg syndrome type 4A (WS4A) [MIM:277580]; also known as Waardenburg-Shah syndrome. WS4A is characterized by the association of Waardenburg features (depigmentation and deafness) and the absence of enteric ganglia in the distal part of the intestine (Hirschsprung disease).
Defects in EDNRB are the cause of Hirschsprung disease type 2 (HSCR2) [MIM:600155]; also known as aganglionic megacolon (MGC). HSCR2 is a congenital disorder characterized by absence of enteric ganglia along a variable length of the intestine. It is the most common cause of congenital intestinal obstruction. Early symptoms range from complete acute neonatal obstruction, characterized by vomiting, abdominal distention and failure to pass stool, to chronic constipation in the older child.
Defects in EDNRB are the cause of ABCD syndrome (ABCDS) [MIM:600501]. ABCD syndrome is an autosomal recessive syndrome characterized by albinism, black lock at temporal occipital region, bilateral deafness, aganglionosis of the large intestine and total absence of neurocytes and nerve fibers in the small intestine.

Similarity:
Belongs to the G-protein coupled receptor 1 family. Endothelin receptor subfamily. EDNRB sub-subfamily.

Gene ID:
1910

Database links:

Entrez Gene: 408082 Chicken

Entrez Gene: 281750 Cow

Entrez Gene: 1910 Human

Entrez Gene: 13618 Mouse

Entrez Gene: 100009477 Rabbit

Entrez Gene: 50672 Rat

Omim: 131244 Human

SwissProt: P28088 Cow

SwissProt: P24530 Human

SwissProt: P48302 Mouse

SwissProt: P35463 Pig

SwissProt: Q9N0W7 Rabbit

SwissProt: P21451 Rat

Unigene: 487 Cow

Unigene: 82002 Human

Unigene: 229532 Mouse

Unigene: 27603 Pig

Unigene: 6857 Rabbit

Unigene: 11412 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.